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Annals of Surgical Oncology
Published in: Annals of Surgical Oncology 8/2015

01-08-2015 | Urologic Oncology

Cytoreductive Surgery Plus Hyperthermic Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei Arising from Urachus

Authors: Yang Liu, MD, Haruaki Ishibashi, MD, PhD, Masamitsu Hirano, MD, PhD, Kazuyoshi Takeshita, MD, PhD, Akiyoshi Mizumoto, MD, PhD, Masumi Ichinose, MD, PhD, Eisei Nishino, MD, PhD, Ippei Kashu, MD, PhD, Yoshihiro Yamamoto, MD, PhD, Paul H. Sugarbaker, MD, FACS, FRCS, Yutaka Yonemura, MD, PhD

Published in: Annals of Surgical Oncology | Issue 8/2015

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Abstract

Background

Pseudomyxoma peritonei (PMP) is a rare locoregional disease characterized by disseminated intraperitoneal mucinous tumors. However, little is known about PMP from urachal neoplasm as a result of its rarity.

Methods

A total of 9 patients with PMP of urachal origin were treated by cytoreductive surgery (CRS) plus hyperthermic intraperitoneal chemotherapy (HIPEC) in our institution. All specimens of surgeries were submitted for pathologic examination. Representative slides of tumors and normal urachus were submitted for immunohistochemical staining.

Results

Four patients were men; the median age was 48 years (range 27–65 years). Initial radiologic examination of all patients showed a cystic tumor located between posterior aspect of umbilicus and the dome of urinary bladder, with or without leaking mucin. Complete CRS and HIPEC were performed in all patients. Until the latest follow-up, local recurrence occurred in 1 patient. Other 8 patients had a median disease-free survival of 27.5 months. Primary urachal tumors of 9 cases were all mucinous adenocarcinoma. Six patients had low-grade mucinous carcinoma peritonei, and 3 patients had high-grade mucinous carcinoma peritonei. Signet ring cells were noted in 4 patients. All tumor specimens of 9 patients were diffuse positive for CK-20, CDX-2, MUC-2, and MUC-5AC, and were variant positive for CK-7.

Conclusions

PMP arising from urachus comes from neoplastic cells with development of intestinal-type mucinous neoplasm. It shares a similar pathophysiology as PMP from appendix. CRS including total urethrectomy, partial cystectomy, and peritonectomy plus HIPEC can be considered as a new option of treatment for PMP originating from urachus.
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Metadata
Title
Cytoreductive Surgery Plus Hyperthermic Intraperitoneal Chemotherapy for Pseudomyxoma Peritonei Arising from Urachus
Authors
Yang Liu, MD
Haruaki Ishibashi, MD, PhD
Masamitsu Hirano, MD, PhD
Kazuyoshi Takeshita, MD, PhD
Akiyoshi Mizumoto, MD, PhD
Masumi Ichinose, MD, PhD
Eisei Nishino, MD, PhD
Ippei Kashu, MD, PhD
Yoshihiro Yamamoto, MD, PhD
Paul H. Sugarbaker, MD, FACS, FRCS
Yutaka Yonemura, MD, PhD
Publication date
01-08-2015
Publisher
Springer US
Published in
Annals of Surgical Oncology / Issue 8/2015
Print ISSN: 1068-9265
Electronic ISSN: 1534-4681
DOI
https://doi.org/10.1245/s10434-014-4336-8

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